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doi: 10.1074/jbc.m610826200

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Impact of ATXN3 Misfolding on the Subcellular Compartments: Mitochondria and ER Wild-type and mutant ATXN3 proteins have also been identified in the mitochondrial protein fraction of cells [138, 139], suggesting that mitochondrial function may be impaired as a result of ATXN3 misfolding and aggregation

Serious adverse effects are rare but have been reported, particularly with IV administration of thiamine